Cryopyrin-Associated Periodic Syndromes (CAPS)

Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare genetic autoinflammatory diseases. Recurrent episodes of fever, skin rash, and joint pain characterize them.

What is CAPS?

CAPS is a term that encompasses three conditions: Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal-Onset Multisystem Inflammatory Disease (NOMID). These conditions share many common symptoms and are caused by mutations in the same gene. Duration of most attacks is less than a day.

Causes of CAPS

CAPS are caused by mutations in the NLRP3 gene, which provides instructions for making a protein that helps regulate inflammation in the body. Mutations in the NLRP3 gene produce an abnormal protein, leading to uncontrolled inflammation and the symptoms of CAPS.

Symptoms of CAPS

The most common symptoms of CAPS include:

  • Recurrent fevers
  • Skin rash (mainly if it happens when cold)
  • Joint pain
  • Eye inflammation
  • Hearing loss (in some cases)
  • Overwhelming fatigue

Symptoms usually begin in infancy or early childhood, but in some cases, they may not appear until later in life.

Diagnosis of CAPS

Diagnosing CAPS can be challenging due to the variability in symptoms and their episodic nature. Diagnosis is typically based on clinical signs and may be confirmed by genetic testing to identify mutations in the NLRP3 gene.

Treatment of CAPS

While there is currently no cure for CAPS, treatments are available to help manage symptoms and prevent complications. Treatment often involves medications that reduce inflammation and suppress the immune system.

Living with CAPS

Living with CAPS can be challenging, but with the proper treatment and support, most individuals with CAPS can lead healthy and productive lives. Regular follow-ups with healthcare providers, a healthy lifestyle, and a strong support network can significantly improve the quality of life for individuals with CAPS.

If you or a loved one has been diagnosed with CAPS, remember you are not alone. Join our Community to connect with others who understand what you’re going through.

Tisztelt Szülő/ Háziorvos Kolléga!

Ha Ön úgy gondolja, hogy visszatérő lázas gyermekénél/betegénél autoinflammatórikus betegség merül fel, itt tud ambuláns kivizsgálásra időpontot kérni:

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Dear Parent or Health Care Professional,

If you suspect that a child or patient with recurrent fever may have an autoinflammatory disease, you can schedule an outpatient examination here:

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